The development of colon innervation in trisomy 16 mice and Hirschsprung's disease

World J Gastroenterol. 2001 Feb;7(1):16-21. doi: 10.3748/wjg.v7.i1.16.


Aim: To study the colon innervation of trisomy 16 mouse, an animal model for Down's syndrome, and the expression of protein gene product 9.5 (PGP 9.5) in the stenosed segment of colon in Hirschsprung's disease (HD).

Methods: Trisomy 16 mouse breeding;cytogenetic analysis of trisomy 16 mice; and PG P9.5 immunohistochemistry of colons of trisomy 16 mice and HD were carried out.

Results: Compared with their normal littermates, the nervous system of colon in trisomy 16 mice was abnormally developed. There existed developmental delay of muscular plexuses of colon, no submucosal plexus was found in the colon, and there was 5mm aganglionic bowel aparting from the anus in trisomy 16 mice. The mesentery nerve fibers were as well developed as shown in their normal littermates. Abundant proliferation of PGP 9.5 positive nerve fibers was revealed in the stenosed segment of HD colon.

Conclusion: Trisomy 16 mice could serve as an animal model for Hirschsprung's disease for aganglionic bowel in the distal part of colon. Abundant proliferation of PGP 9.5 positive fibers resulted from extrinsic nerve compensation, since no ganglionic cells were observed in the stenosed segment of the colon in HD. HD has a genetic tendency.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Colon / abnormalities*
  • Colon / innervation*
  • Constriction, Pathologic
  • Disease Models, Animal
  • Down Syndrome / genetics
  • Female
  • Hirschsprung Disease / genetics*
  • Hirschsprung Disease / pathology
  • Immunohistochemistry
  • Mice
  • Mice, Mutant Strains
  • Myenteric Plexus / abnormalities*
  • Nerve Fibers / chemistry
  • Pregnancy
  • Submucous Plexus / abnormalities*
  • Thiolester Hydrolases / analysis
  • Trisomy
  • Ubiquitin Thiolesterase


  • Thiolester Hydrolases
  • Ubiquitin Thiolesterase